maple syrup urine disease/proline

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NakalaMajaribio ya klinikiHati miliki

Chagua aina ya aina

3 matokeo

Azetidine-2-carboxylic acid contaminated dietary proline as a cause of urinary excretion of 4-amino-2-(S-cysteinyl)butyric acid in patients on oral treatment with a synthetic diet.

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Ingia / Ingia

Three children with branched-chain ketoaciduria (maple syrup urine disease) were found to excrete an abnormal amino acid when they were on an artificial diet. This substance was identified as 4-amino-2-(S-cysteinyl)butyric acid with the use of column liquid chromatography, gas chromatography--mass

Definition of the mutation responsible for maple syrup urine disease in Poll Shorthorns and genotyping Poll Shorthorns and Poll Herefords for maple syrup urine disease alleles.

Watumiaji waliosajiliwa tu ndio wanaweza kutafsiri nakala

Ingia / Ingia

The organisation of the E1alpha subunit of bovine branched-chain alpha-keto acid dehydrogenase gene was established. c DNA was cloned from Poll Shorthorn x Poll Hereford calves affected with Maple Syrup Urine Disease to identify the mutation responsible for the disease in Poll Shorthorns. Clones

Molecular studies of mammalian branched-chain alpha-keto acid dehydrogenase complexes: domain structures, expression, and inborn errors.

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Ingia / Ingia

We have cloned cDNAs encoding the E1b-alpha, E1b-beta, and E2b subunits of the bovine and human branched-chain alpha-keto acid complexes. The deduced primary structures indicate that the mammalian E2b contains a lipoyl-bearing, an E3-binding, and an inner core domain that are linked in series by two

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Herbal & Natural Medicine

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